Clinical Picture

Depending on the type of the disease wounds heal with or without scars. In cases when scars occur it comes to coalescence, most often of the fingers on the hands and feet, and the lower mobility of the individual joints. Often the fingers on hands completely grow together and looks like glove without fingers. The walk is very difficult so many patients need a wheelchairs.

Since changes may affect mucous membranes the disease can lead to the tongue coalescence and narrowing of the esophagus. This cause severe difficulties with swallowing of the solid food, even fluid and difficult opening of the mouth. Apart from the aforementioned, feeding is also hampered by soreness in the mouth and poor quality of the teeth due to the illness itself and the difficult maintenance of the hygiene, which causes very early dental caries.

The rhinitis also occurs in the eyes and lead to lacrimation and pain and may sometimes result in permanent visual impairment. Nutritional requirements, especially protein, iron, and vitamins, are increased. On the one hand, there is a great loss of all nutrients and blood through the wounds on the skin and mucous membranes, and on the other hand, larger quantities of these substances are needed due to the need for wound healing.

There is often a problem of constipation due to the pain during it. This leads to retention of bowel movements, and reduction of appetite.

Feeding for these patients is very tedious and difficult, and often there is created a vicious circle of poor food, malnutrition, weakness, constipation and poor wound healing.

People with bullous epidemolysis are suffering from pain. It is the result of painful skin and mucous wounds.

Because of the constant presence of wounds, infections of skin and other organic systems, caused by various bacteria, are common.

Immediate cause of death in a significant percentage of diseased is the development of malignant skin tumors.

Such tumors do not usually occur before the adolescent age, and are characterized by a very rapid growth and early spreading.